Tracheobronchopathia osteochondroplastica (TO) is a rare benign disease of the endobronchial system with nonspecific symptoms and different treatment approaches. We report on a group of patients with TO and discuss their presentation and their treatment modalities. Between 2000 and 2006, the medical records of patients with TO were studied at the Interventional Pulmonary Unit of the Tracheal Disease Research Center at Masih Daneshvary Hospital, a referral center for respiratory diseases in Tehran, Iran. We analyzed and studied patients' demographics, symptom presentation, radiographic appearance, bronchoscopic findings, and their subsequent treatment. Of the 8,760 patients who underwent flexible bronchoscopy (FOB) at our center over 6 years, 10 were diagnosed with TO; their median age was 51 years (range = 16-68 years) and 6 were men. Computerized tomography (CT) revealed mucosal irregularity and calcified nodules. Histopathologic examination of tissue biopsy through bronchoscopy was used for definitive diagnosis of TO. Bronchoscopy findings showed the endobronchial lesions to be firm and glossy. Cartilaginous nodules were present in central airways. Treatment included endobronchial Nd:YAG laser photoevaporation (LPE) (n = 10), coring of the lesions with the tip of the rigid bronchoscope (n = 4), and endobronchial stent placement (n = 1). Symptoms were considerably relieved in six cases but there was no significant improvement noted in three patients. One patient died after 6 years of treatment and follow-up. TO is a rare disease and the diagnosis should be suspected based on CT findings or bronchoscopic examination of the airways. Histopathologic examination is required for confirmation of diagnosis. Treatment is palliative and it includes LPE, coring through a rigid bronchoscope, and endobronchial stent placement.